Final answer:
Patients with Mullerian Agenesis typically present with normal secondary sexual characteristics but have an absence of the uterus and the upper two-thirds of the vagina. They go through puberty, develop secondary sexual characteristics, but do not menstruate and cannot conceive naturally. This condition is due to the improper development of the Mullerian ducts during fetal development.
Step-by-step explanation:
Mullerian Agenesis Presentation
Patients with Mullerian Agenesis, also known as Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, present with a particular set of characteristics. These patients usually have normal secondary sexual characteristics such as breast development and pubic hair but will have an absence of the uterus and the upper two-thirds of the vagina. Unlike other conditions that affect sexual development, such as androgen insensitivity syndrome, individuals with Mullerian Agenesis will have normally functioning ovaries and will experience puberty and develop secondary sexual characteristics. However, because they lack a uterus, they do not menstruate and are unable to conceive a child, leading to primary amenorrhea. They are usually raised as females and have a female gender identity.
In the context of female fetal development, the internal reproductive structures such as the uterus, uterine tubes, and part of the vagina in females are formed from the Müllerian ducts. If these ducts fail to develop properly, as seen in Mullerian Agenesis, it results in the characteristic absence of certain reproductive structures. It is important to differentiate this condition from other disorders that can lead to ambiguous genitalia, virilization, or excessive hair growth (hirsutism), as these symptoms are not typically associated with Mullerian Agenesis.